Decompensated Liver Disease

Last updated 10th October 2022

Always complete the tests outlined in the ‘’BASL / BSG Decompensated Cirrhosis Care Bundle’’ in the first 24 hours of admission. Consider any delay in treatment of infection (e.g. SBP) increases mortality . If suspected SBP (e.g. rebound tenderness) then treat empirically.

Definition

Decreased synthetic liver function (albumin, prothrombin time) is hepatic impairment. In decompensation, in addition jaundice (decreased excretory liver function), encephalopathy, ascites or hepatorenal syndrome develop on the background of cirrhosis – best thought of as acute on chronic liver failure.

Underlying Causes of the Chronic Liver Disease

Alcoholic Liver disease (ALD) accounts for 80 % of cases in Scotland
The other most common causes are include metabolic syndrome associated fatty liver disease (MAFLD), blood born viruses (BBV: HBV and HCV), less common causes are: autoimmune hepatitis, primary biliary cholangitis, haemochromatosis, alpha1-antitrypsin deficiency. Often there are several contributing (chronic / acute) factors.

Triggers That Lead to Decompensation

Alcohol (but sometimes patients decompensate on stopping drinking)
Sepsis – remember to consider bacterial peritonitis – any minute that treatment start later risks lives!
Dehydration – common including diuretic treatment for ascites
Drugs – especially benzodiazepines in withdrawal regimens & opiates
Electrolyte upset – especially hyponatraemia
GI bleeding – need not be overt eg small leaks from portal hypertensive gastropathy (PHG) – remember, in about 50 % of patients bleeding is triggered by infection therefore everybody with a bleed needs to be treated with antibiotics.
Constipation (with build up of toxins – lactulose is the only effective treatment)
Diarrhoea (can lead to dehydration)
Development of hepatocellular carcinoma

Clinical Features

Jaundice, encephalopathy (click to view section on Acute Liver Failure), worsening of ascites or hepatorenal syndrome, usually but not always with stigmata of chronic liver disease.
Stigmata of CLD are most likely to be alcohol related and include spider naevi, liver palms, parotid swelling, gynaecomastia (due to decreased metabolism of male sex hormones or spironolactone side effect)
Other signs of portal hypertension, which are splenomegaly and oesophageal varices, may also be present and need to be checked for.

Investigations

Routine – FBC, U&E, liver biochemistry (AST and ALT, albumin and prothrombin time), CXR, blood and urine cultures.
Abdominal ultrasound with Doppler of the portal vein to look for complications of liver disease: liver tumours, define biliary tree (? Obstruction), establish spleen size, look for ascites and determine patency and flow speed of the portal vein and hepatic veins (Budd Chiari Syndrome).
HBV/HCV viral screen, immunoglobulins and liver autoantibodies, ferritin and transferrin saturation, caeruloplasmin if patient less than 50 years old, alpha1-antitrypsin, lipid profile to help establish an underlying cause.
AFP if not recently performed, but note that many HCCs don’t secrete AFP.
Tap ascites if present for WCC, protein and culture (see above), but treat early if suspected infection. Any delay in treatment risks lives.
Always assess for alcohol excess and refer to the alcohol team if indicated.

Management

Encephalopathy (grade 1: subclinical, grade 2: liver flap, grade 3: stupor, grade 4: coma)
1. identify and treat precipitating factors;
2. normal protein and high calorie diet (do not protein restrict).
3. stop diuretics if serum Na <125 mmol/l.
4. stop sedatives.
5. Always treat with lactulose. If grade 3 or 4 give 100 ml Lactulose stat on admission (if in coma might need NG tube). This acidifies the bowel content (decreasing toxin producing bacteria) and clears bacteria from the bowel, otherwise start Lactulose 30 ml four times daily then titrated to give at least 3 soft stools daily (if the bowels haven’t opened 3 times by 17:00 an additional dose needs to be given). The dose is not relevant, it is the number of bowel motions that is relevant, therefore stool chart is vital. Only lactulose has this effect on pH and therefore no all-other laxatives should be stopped. Enemas should only be given once (e.g., 2 phosphate enemas) in severe rectal / sigmoid impaction) This does not improve outcome, it only makes the lactulose work.
Ascites – no added salt low sodium diet with Spironolactone with or without Frusemide (if leg oedema); click here for Ascites.
Renal Failure – catheterise, stop nephrotoxic drugs, rehydrate, treat sepsis; click here for link to Hepatorenal Syndrome (consider terlipressin).
Coagulopathy: Be aware that this is a marker of severely decreased synthetic liver function rather than a marker of increased bleeding risk. As a result of decreased function patients have a decrease in anti-coagulant and pro-coagulant factors. These are re-balanced at a very low level with a tendency of patients being rather pro-coagulant (and a higher risk of developing blood clots). They are also much more likely to develop disseminated intravascular coagulation (DIC). Without bleeding – do not give Vitamin K. This makes it much more difficult to determine severity of liver disease, decision for transplant (e.g in POD). If the patient is in liver failure, it can’t produce clotting factors with or without Vitamin K (but malnurised patients might be generally vitamin deficient). Do not give blood products (i.e. fresh frozen plasma, factor concentrates) unless bleeding is a problem. If coagulopathy with bleeding – Discuss with haematology or liver consultant. Will require FFP, also platelets if <20,000.
Sepsis – give antibiotics at earliest sign of sepsis and do a sepsis screen (CXR, urine dip, tap ascites (consider iatrogenic bacterial peritonitis if recent tap or drain especially if leakage was not managed correctly).
Remember there are other causes of confusion in a patient with chronic liver disease eg. Wernicke’s, intracranial bleed, dementia, small vessel disease,…
Escalation – If develops grade 2 encephalopathy or higher / GCS falling move to ward 8 or CCU / ITU as indicated. Continue lactulose via NG tube, discuss with liver team / ITU and if appropriate with Edinburgh Liver Unit (will give advice even if transfer is not appropriate).

Outcome – Child-Pugh Classification

2 clinical features and 3 biochemical parameters may conveniently be used to predict outcome as follows:

Clinical/Laboratory Findings	Score 1	Score 2	Score 3
Encephalopathy	None	Mild (grade 1-2)	Severe (grade 3-4)
Acites	None	Mild/Slight	Moderate/Large
Bilirubin (micromol/L)	<34	34 - 51	>51
Albumin (g/L)	≥35	28 - 35	<28
INR	<1.3	1.3 - 1.5	>1.5

Total Points	Child-Pugh Class	Survival
5 - 6	A	80-% 2 year survival
7 - 9	B	60% 2 year survival
10 - 15	C	30% 2 year survival